ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease

Katerina M Antoniou; Oliver Distler; Ana-Maria Gheorghiu; Catharina C Moor; Jens Vikse; Nikoleta Bizymi; Ilaria Galetti; Graham Brown; Elena Bargagli; Yannick Allanore; Tamera J Corte; Philippe Dieudé; Vincent Cottin; Benjamin A Fisher; Aurelie Fabre; Jon T Giles; Michael Kreuter; Ingrid E Lundberg; Venerino Poletti; Britta Maurer; Elisabetta A Renzoni; Ulf Müller-Ladner; Mary E Strek; Nicola Sverzellati; Paul Studenic; Jibril Mohammed; Blin Nagavci; Tanja Stamm; Thomy Tonia; Bruno Crestani; Anna-Maria Hoffmann-Vold

doi:10.1183/13993003.02533-2024

. 2026 Jan 22;67(1):2402533. doi: 10.1183/13993003.02533-2024

ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease

Katerina M Antoniou ^1,³⁶, Oliver Distler ^2,³⁶, Ana-Maria Gheorghiu ³, Catharina C Moor ⁴, Jens Vikse ^5,^6,⁷, Nikoleta Bizymi ¹, Ilaria Galetti ⁸, Graham Brown ^9,^†, Elena Bargagli ¹⁰, Yannick Allanore ¹¹, Tamera J Corte ¹², Philippe Dieudé ¹³, Vincent Cottin ¹⁴, Benjamin A Fisher ^15,¹⁶, Aurelie Fabre ^17,¹⁸, Jon T Giles ¹⁹, Michael Kreuter ²⁰, Ingrid E Lundberg ^21,²², Venerino Poletti ²³, Britta Maurer ²⁴, Elisabetta A Renzoni ²⁵, Ulf Müller-Ladner ²⁶, Mary E Strek ²⁷, Nicola Sverzellati ²⁸, Paul Studenic ^21,²⁹, Jibril Mohammed ³⁰, Blin Nagavci ³¹, Tanja Stamm ³², Thomy Tonia ³³, Bruno Crestani ^34,³⁶, Anna-Maria Hoffmann-Vold ^2,^35,^36,^✉

¹Department of Respiratory Medicine, Laboratory of Molecular and Cellular Pneumonology, Medical School, University of Crete, Heraklion, Greece

²Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland

³Internal Medicine and Rheumatology Department, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

⁴Centre for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands

⁵Department of Clinical Science, University of Bergen, Bergen, Norway

⁶Department of Rheumatology, Stavanger University Hospital, Stavanger, Norway

⁷Department of Chemistry, Bioscience and Environmental Engineering, University of Stavanger, Stavanger, Norway

⁸PARE/EULAR

⁹ELF/EU-IPFF

¹⁰Respiratory Diseases Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy

¹¹Université Paris Cité, AP-HP, Hôpital Cochin, Rheumatology, Paris, France

¹²Department of Respiratory Medicine, Royal Prince Alfred Hospital and University of Sydney, Sydney, Australia

¹³Université Paris Cité, AP-HP, Hôpital Bichat-Claude Bernard, Rheumatology, Paris, France

¹⁴Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, INRAE, ERN-LUNG, Claude Bernard University Lyon 1, Lyon, France

¹⁵Rheumatology Research Group, Institute of Inflammation and Ageing, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK

¹⁶National Institute for Health Research Birmingham Biomedical Research Centre and Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK

¹⁷Department of Pathology, St Vincent's University Hospital, Dublin, Ireland

¹⁸School of Medicine, University College Dublin, Dublin, Ireland

¹⁹Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, CA, USA

²⁰Mainz Center for Pulmonary Medicine, Department of Pneumology, ZfT, Mainz University Medical Center and Department of Pulmonary, Critical Care and Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany

²¹Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden

²²Department of Gastro, Dermatology and Rheumatology, Theme Inflammation and Aging, Karolinska University, Stockholm, Sweden

²³DIMEC-Bologna University/University Morgagni Hospital Medical Specialities Department, Forlì, Italy

²⁴Department of Rheumatology and Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland

²⁵Interstitial Lung Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK

²⁶Department of Rheumatology and Clinical Immunology, Justus-Liebig-University Giessen, Campus Kerckhoff, Bad Nauheim, Germany

²⁷Section of Pulmonary and Critical Care Medicine, Department of Medicine, University of Chicago, Chicago, IL, USA

²⁸Scienze Radiologiche, Department of Medicine and Surgery, University of Parma, Parma, Italy

²⁹Medical University of Vienna, Department of Internal Medicine 3, Division of Rheumatology, Wien, Austria

³⁰Department of Physiotherapy, Bayero University Kano and Aminu-Kano Teaching Hospital, Kano, Nigeria

³¹Institute for Evidence in Medicine, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg im Breisgau, Germany

³²Medical University of Vienna, Section for Outcomes Research, Center for Medical Statistics, Informatics, and Intelligent Systems, Wien, Austria

³³Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland

³⁴Université Paris Cité, AP-HP, Hôpital Bichat-Claude Bernard, Respiratory Medicine, Paris, France

³⁵Department of Rheumatology, Oslo University Hospital, Oslo, Norway

³⁶Equally contributing authors

^†Deceased

^✉

Corresponding author: Anna-Maria Hoffmann-Vold (a.m.hoffmann-vold@medisin.uio.no)

PMCID: PMC12824641 PMID: 40907995

Abstract

Background

Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care.

Methods

A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies. For both PICO and narrative questions, the Evidence to Decision framework was used to formulate the recommendations.

Results

The task force committee concluded with recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). In four narrative questions, regarding screening and assessment of risk for ILD progression in MCTD, SjD and SLE and one PICO question regarding pirfenidone in CTD-ILD other than RA-ILD, the task force had insufficient evidence to support recommendations. Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice.

Conclusions

We provide practical guidance by evidence-based recommendations to clinicians for each of the CTDs. In many cases there is low certainty or absence of evidence and we encourage further research to fill these gaps.

Shareable abstract

This ERS/EULAR clinical practice guideline offers evidence-based recommendations on the screening, diagnosis, monitoring and treatment of CTD-ILDs. It emphasises the importance of further research in areas where evidence is lacking or certainty is low. https://bit.ly/4mX1bPB

Introduction

Interstitial lung disease (ILD) is one of the most frequent manifestations in connective tissue diseases (CTDs) and is associated with high morbidity and mortality. International collaborations by pulmonology and rheumatology societies including patient research partners are important to develop guidelines with evidence-based approaches for screening, diagnosis, monitoring and treatment of ILD in CTDs for optimised management in clinical practice [1–3].

A collaborative effort between the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR) led to the formation of a task force aimed at developing clinical practice guidelines addressing these critical clinical aspects of CTD-ILD.

Methods

Task force composition

The task force was approved by both societies (ERS and EULAR) and chaired by four experts (two from each society) and consisted of nine senior pulmonologists, nine rheumatologists, one radiologist and one histopathologist, and three early career members/fellows (supplementary file 1). Two patient representatives participated as full task force members in the guideline development and participated in all steps and meetings. Two more early career individuals contributed to the systematic reviews. Two ERS methodologists and one EULAR methodologist overviewed the guideline development process. The task force members were divided into four working groups covering screening, diagnosis/assessment of severity, monitoring and treatment, respectively. All potential conflicts of interest were disclosed by the task force members and managed according to society policies, and compliance was monitored by the chairs. The panel met numerous times in person and virtually. The guideline was developed following the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach as stated in the “memorandum of understanding” agreed on and signed by both ERS and EULAR.

Formulation of questions and selection of outcomes and their importance

Since CTD is a heterogeneous group of disorders, we assessed each question in four disease groups: systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIM), and other CTDs, including Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). We included rheumatoid arthritis (RA) under the umbrella term CTD in this guideline. All task force members formulated PICO (Patients, Intervention, Comparison, Outcomes) (tables 1a and 1b) as well as narrative questions (tables 1a and 1c) [4], covering the key issues separately for each of the four pre-defined disease groups. For the PICO questions, the outcomes were rated as critical, important or not important for clinical decision-making, through anonymous online voting of all task force members, including the patient representatives [5]. Research priorities were formulated (table 1d). We only assessed critical or important outcomes.

TABLE 1a.

Overview of recommendations grouped by screening, diagnosis, monitoring and treatment for patients with connective tissue disease (CTD)-associated interstitial lung disease (ILD) and rheumatoid arthritis (RA)-associated ILD

Recommendations		Strength of recommendation	Level/certainty of evidence
Screening
1) PICO 1–11	We recommend against replacing HRCT with pulmonary function tests for screening of ILD in patients with SSc, RA, IIM (S, L) and other CTDs (S, VL). We suggest not to replace HRCT with lung ultrasound for screening of ILD in patients with SSc, RA, IIM and other CTDs (C, L/VL).	S/C	L/VL
2) NQ 1–5	We recommend that all patients with SSc and MCTD and IIM patients with risk factors should be screened (S, L); and suggest that all patients with RA and SjD with risk factors, and IIM patients without risk factors (C, L) could be screened for ILD.	S/C	L
Diagnosis
3) NQ 8–10	We suggest performing a global assessment of all risk factors of ILD progression in patients with SSc, RA and IIM to identify patients at higher risk of ILD progression and death.	C	L
4) NQ 12–13	We suggest that BAL could be used in patients with any CTD-ILD at the time of diagnosis in cases where there is suspicion of infection or to exclude alternative diagnoses. We suggest that lung biopsy should not play a role for diagnosis.	C	VL
5) NQ 14–19	We suggest using the 6MWT in patients without physical limitations and PROMs to assess severity and/or prognosis of ILD in any CTD-ILD patients.	C	L/VL
Monitoring
6) NQ 20–27	We suggest repeating PFTs every 3–6 months during the first years, and at least every 6–12 months thereafter. We suggest regularly repeating HRCT after 1–2 years in patients with SSc-ILD, RA-ILD and other CTD-ILD, and after 3–6 months in IIM-ILD, particularly in those at higher risk of progression. We suggest repeating PFTs and HRCT in case of suspected progression in any CTD-ILD patient.	C	L/VL
Treatment
7) PICO 12–15	We recommend using tocilizumab in a subgroup (S, M) and suggest using MMF, rituximab (C, VL), and cyclophosphamide (C, L) in patients with SSc-ILD.	S/C	M/L/VL
8) PICO 16	We recommend using immunosuppressive treatment in patients with IIM-ILD.	S	VL
9) PICO 17–18	We suggest using immunosuppressive treatment in patients with RA-, SjD-, MCTD- and SLE-ILD.	C	VL
10) PICO 19–20	We suggest using nintedanib in SSc-ILD (C, M) and in any CTD-ILD (C, VL) patient with progressive pulmonary fibrosis.	C	M/L/VL
11) PICO 21	We suggest using pirfenidone in patients with RA-ILD with a UIP pattern.	C	VL
12) PICO 23	We suggest using combination therapy with nintedanib and MMF in patients with SSc-ILD.	C	VL
13) PICO 24	We suggest using combination therapy with immunosuppressants including glucocorticoids in patients with IIM-ILD.	C	VL
14) PICO 25	We suggest treating patients with any CTD-ILD with a combination of immunosuppressants or, in the presence of progressive pulmonary fibrosis, with a combination of an immunosuppressant and nintedanib.	C	VL
15) NQ 28	We suggest using the inclusion criteria of RCTs to guide treatment decisions for CTD-ILD.	C	VL

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PICO: Patients, Intervention, Comparison, Outcomes; NQ: narrative question; S: strong recommendation; C: conditional recommendation; M: moderate evidence; L: low evidence; VL: very low evidence; HRCT: high-resolution computed tomography; SSc: systemic sclerosis; IIM: idiopathic inflammatory myopathies; SjD: Sjögren disease; BAL: bronchoalveolar lavage; 6MWT: 6-min walk test; PROM: patient-reported outcome measure; PFT: pulmonary function test; MMF: mycophenolate mofetil; MCTD: mixed connective tissue disease; SLE: systemic lupus erythematosus; UIP: usual interstitial pneumonia; RCT: randomised controlled trial.

TABLE 1b.

Task force recommendations for PICO (Patients, Intervention, Comparison, Outcomes) questions for connective tissue disease (CTD)-associated interstitial lung disease (ILD) and rheumatoid arthritis (RA)-associated ILD

PICO question	Recommendations
Screening
1) Should PFTs (FVC, D_LCO) be used as a replacement for HRCT to screen for ILD in patients diagnosed with SSc?	We recommend against replacing HRCT with PFTs for screening of ILD in patients with SSc (strong recommendation against, low certainty of evidence).
2) Should PFTs (FVC, D_LCO) be used as a replacement for HRCT to screen for ILD in patients diagnosed with RA?	We recommend against replacing HRCT with PFTs for screening of ILD in patients with RA (strong recommendation against, low certainty of evidence).
3) Should PFTs (FVC, D_LCO) be used as a replacement for HRCT to screen for ILD in patients diagnosed with IIM?	We recommend against replacing HRCT with PFTs for screening of ILD in patients with IIM (strong recommendation against, low certainty of evidence).
4) Should PFTs (FVC, D_LCO) be used as a replacement for HRCT to screen for ILD in patients diagnosed other CTDs?	We recommend against replacing HRCT with PFTs for screening of ILD in patients with other CTDs (strong recommendation against, very low certainty of evidence; no studies included).
5) Should LUS be used as a replacement for HRCT to screen for ILD in patients diagnosed with CTD?	We suggest not to replace HRCT with LUS for screening of ILD in patients with CTDs (conditional recommendation against, low certainty of evidence).
6) Should LUS with an extended protocol be used as a replacement for HRCT to screen for ILD in patients diagnosed with CTD?	We suggest not to replace HRCT with LUS with an extended protocol for screening of ILD in patients with CTDs (conditional recommendation against, very low certainty of evidence).
7) Should LUS with a short protocol be used as a replacement for HRCT to screen for ILD in patients diagnosed with CTD?	We suggest not to replace HRCT with LUS with a short protocol for screening of ILD in patients with CTDs (conditional recommendation against, low certainty of evidence).
8) Should LUS with an extended protocol be used as a replacement for HRCT to screen for ILD in patients diagnosed with SSc?	We suggest not to replace HRCT with LUS with an extended protocol for screening of ILD in patients with SSc (conditional recommendation against, very low certainty of evidence).
9) Should LUS with a short protocol be used as a replacement for HRCT to screen for ILD in patients diagnosed with SSc?	We suggest not to replace HRCT with LUS with a short protocol for screening of ILD in patients with SSc (conditional recommendation against, low certainty of evidence).
10) Should LUS be used as a replacement for HRCT to screen for ILD in patients diagnosed with RA?	We suggest not to replace HRCT with LUS for screening of ILD in patients with RA (conditional recommendation against, low certainty of evidence).
11) Should LUS be used as a replacement for HRCT to screen for ILD in patients diagnosed with other CTDs?	We suggest not to replace HRCT with LUS for screening of ILD in patients with other CTDs (conditional recommendation against, low certainty of evidence).
Treatment
12) Should MMF versus control be used for patients with SSc-ILD?	We suggest using MMF in patients with SSc-ILD (conditional recommendation, very low certainty of evidence).
13) Should tocilizumab versus control be used for patients with SSc-ILD?	We recommend using tocilizumab in SSc-ILD patients with early diffuse cutaneous SSc and increased inflammatory markers or recent skin fibrosis progression (strong recommendation, moderate certainty of evidence).
14) Should rituximab versus control be used for patients with SSc-ILD?	We suggest using rituximab in patients with SSc-ILD (conditional recommendation, very low certainty of evidence).
15) Should cyclophosphamide versus control be used for patients with SSc-ILD?	We suggest using cyclophosphamide in patients with SSc-ILD (conditional recommendation, low certainty of evidence).
16) Should immunosuppressive treatment versus control be used for patients with IIM-ILD?	We recommend using immunosuppressive treatment in patients with IIM-ILD (strong recommendation, very low certainty of evidence).
17) Should immunosuppressive treatment versus control be used for patients with RA-ILD?	We suggest using immunosuppressive treatment in patients with RA-ILD (conditional recommendation, very low certainty of evidence).
18) Should immunosuppressive treatment versus control be used for patients with other CTD-ILD?	We suggest using immunosuppressive treatment in patients with SjD, MCTD and SLE-ILD (conditional recommendation, very low certainty of evidence; no studies included).
19) Should nintedanib versus control be used for patients with SSc-ILD?	We suggest using nintedanib in patients with SSc-ILD (conditional recommendation, moderate certainty of evidence).
20) Should nintedanib versus control be used for patients with any CTD-ILD?	We suggest using nintedanib in any CTD-ILD patients with progressive pulmonary fibrosis (conditional recommendation, low certainty of evidence).
21) Should pirfenidone versus control be used for patients with RA-ILD?	We suggest using pirfenidone in patients with RA-ILD with a UIP pattern (conditional recommendation, very low certainty of evidence).
22) Should pirfenidone versus control be used for patients with CTD-ILD other than RA-ILD?	No recommendation.
23) Should combination therapy with nintedanib and MMF versus control be used for patients with SSc-ILD?	We suggest using combination therapy with nintedanib and MMF in patients with SSc-ILD (conditional recommendation, low certainty of evidence).
24) Should combination therapy versus control be used for patients with IIM-ILD?	We suggest using combination therapy with immunosuppressants including glucocorticoids in patients with IIM-ILD (conditional recommendation, very low certainty of evidence).
25) Should combination therapy versus control be used for any patient with CTD-ILD?	We suggest treating patients with any CTD-ILD with a combination of immunosuppressants or, in the presence of progressive pulmonary fibrosis, with a combination of an immunosuppressant and nintedanib (conditional recommendation, very low certainty of evidence; no studies included).

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PFT: pulmonary function test; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; SSc: systemic sclerosis; IIM: idiopathic inflammatory myopathies; LUS: lung ultrasound; MMF: mycophenolate mofetil; SjD: Sjögren disease; MCTD: mixed connective tissue disease; SLE: systemic lupus erythematosus; UIP: usual interstitial pneumonia.

TABLE 1c.

Task force recommendations for narrative questions for connective tissue disease (CTD)-associated interstitial lung disease (ILD) and rheumatoid arthritis (RA)-associated ILD

Narrative question	Recommendations
Screening
1) Which patients with SSc should be screened for ILD?	We recommend that all patients with SSc should be screened for ILD using HRCT (strong recommendation, low certainty of evidence stemming from narrative review).
2) Which patients with RA should be screened for ILD?	We suggest that patients with RA and risk factors for ILD including older age, smoking history, elevated rheumatoid factor, anti-CCP antibodies, increased inflammatory markers, male sex and high articular disease activity could be screened for ILD using HRCT (conditional recommendation, low certainty of evidence stemming from narrative review).
3) Which patients with IIM should be screened for ILD?	We recommend that patients with IIM and risk factors, including anti-synthetase syndrome, clinically amyopathic dermatomyositis, presence of mechanic's hands, arthritis and certain myositis-associated autoantibodies (anti-synthetase, anti-MDA-5 and anti-Ro52 antibodies), should be screened for ILD using HRCT (strong recommendation, low certainty of evidence stemming from narrative review). We suggest that most patients with IIM without risk factors could be screened for ILD using HRCT, except patients with inclusion body myositis (conditional recommendation, low certainty of evidence stemming from narrative review).
4) Which patients with MCTD should be screened for ILD?	We recommend that all patients with MCTD should be screened for ILD using HRCT (strong recommendation, low certainty of evidence stemming from narrative review).
5) Which patients with SjD should be screened for ILD?	We suggest that patients with SjD and risk factors including older age, male sex, active extrapulmonary organ involvement and increased inflammatory markers could be screened for ILD using HRCT (conditional recommendation, low certainty of evidence stemming from narrative review).
6) Which patients with SLE should be screened for ILD?	No recommendation.
7) How often should patients with CTD be screened for ILD?	No recommendation.
Diagnosis and monitoring
8) How should patients with SSc be evaluated at baseline to assess risk of ILD progression or death?	We suggest performing a global assessment of all risk factors of ILD progression, including HRCT, PFTs (FVC and D_LCO), autoantibodies (particularly ATA-I), African American ethnicity, skin involvement (extent and progression) and markers of inflammation (CRP and/or ESR) to identify SSc-ILD patients at higher risk of ILD progression and death (conditional recommendation, low certainty of evidence stemming from narrative review).
9) How should patients with RA be evaluated at baseline to assess risk of ILD progression or death?	We suggest performing a global assessment of all risk factors of ILD progression, including UIP pattern and extent of ILD on HRCT, and PFTs (FVC and D_LCO) to identify patients with RA-ILD at higher risk of ILD progression and death (conditional recommendation, low certainty of evidence stemming from narrative review).
10) How should patients with IIM be evaluated at baseline to assess risk of ILD progression or death?	We suggest performing a global assessment of all risk factors of ILD progression, including HRCT, PFTs (FVC and D_LCO) and autoantibody profile (anti-MDA-5 antibody, anti-synthetase antibodies, anti-Ro52 antibody) to identify patients with IIM-ILD at high risk of ILD progression and death (conditional recommendation, low certainty of evidence stemming from narrative review).
11) How should patients with other CTDs be evaluated at baseline to assess risk of ILD progression or death?	No recommendation.
12) What is the role of BAL in patients with CTD-ILD at the time of diagnosis?	We suggest that BAL could have a role in patients with CTD-ILD at the time of diagnosis in cases where there is suspicion of infection or to exclude alternative diagnoses (conditional recommendation, very low certainty of evidence stemming from narrative review).
13) What is the role of lung biopsy in patients with CTD-ILD at the time of diagnosis?	We suggest that lung biopsy should not play a role at the time of diagnosis in patients with CTD-ILD (conditional recommendation against, very low certainty of evidence stemming from narrative review).
14) What is the role of the 6MWT in assessing severity and/or prognosis of ILD in patients with SSc?	We suggest using the 6MWT to assess severity and/or prognosis of ILD in SSc-ILD patients (conditional recommendation, low certainty of evidence stemming from narrative review).
15) What is the role of the 6MWT in assessing severity and/or prognosis of ILD in patients with RA?	We suggest using the 6MWT to assess severity and/or prognosis of ILD in RA-ILD patients with limited or no lower limb joint damage or active synovitis (conditional recommendation, very low certainty of evidence stemming from narrative review).
16) What is the role of the 6MWT in assessing severity and/or prognosis of ILD in patients with IIM?	We suggest using the 6MWT to assess severity of ILD in IIM-ILD patients without significant muscle involvement of the lower limbs (conditional recommendation, very low certainty of evidence stemming from narrative review).
17) What is the role of the 6MWT in assessing severity and/or prognosis of ILD in patients with other CTDs?	We suggest using the 6MWT to assess severity and/or prognosis of ILD in patients with other CTD-ILD (conditional recommendation, very low certainty of evidence stemming from narrative review).
18) What is the role of PROMs in assessing severity and/or prognosis of ILD in patients with SSc?	We suggest using PROMs to assess severity of ILD in patients with SSc-ILD (conditional recommendation, low certainty of evidence stemming from narrative review).
19) What is the role of PROMs in assessing severity and/or prognosis of ILD in patients with CTD other than SSc?	We suggest using PROMs to assess severity and/or prognosis of ILD in patients with CTD-ILD other than SSc-ILD (conditional recommendation, very low certainty of evidence stemming from narrative review).
Monitoring
20) In patients with SSc-ILD being followed-up, when should PFTs be repeated?	We suggest repeating PFTs (FVC and D_LCO) every 3–6 months during the first 3–5 years for follow-up in patients with SSc-ILD, and at least every 6–12 months thereafter, and in case of suspected progression (conditional recommendation, low certainty of evidence stemming from narrative review).
21) In patients with RA-ILD being followed-up, when should PFTs be repeated?	We suggest repeating PFTs (FVC, D_LCO and FEV₁ in view of the possible concomitant airway component) every 3–6 months during the first 1–2 years for follow-up in patients with RA-ILD, and at least every 6–12 months thereafter, and in case of suspected progression (conditional recommendation, very low certainty of evidence stemming from narrative review).
22) In patients with IIM-ILD being followed-up, when should PFTs be repeated?	We suggest performing PFTs (FVC and D_LCO) every 3–6 months during the first year for follow-up in patients with IIM-ILD, and at least every 6–12 months thereafter, and in case of suspected progression (conditional recommendation, very low certainty of evidence stemming from narrative review).
23) In patients with other CTD-ILD being followed-up, when should PFTs be repeated?	We suggest performing PFTs (FVC, D_LCO and FEV₁ in view of the possible concomitant airway component) every 3–6 months during the first year for follow-up in patients with SjD-ILD, and at least every 6–12 months thereafter, and in case of suspected progression (conditional recommendation, very low certainty of evidence stemming from narrative review). No recommendation for SLE and MCTD.
24) In patients with SSc-ILD being followed-up, when should HRCT be repeated?	We suggest regularly repeating HRCT after 1–2 years in patients with SSc-ILD, particularly in those at higher risk of progression and in case of suspected progression (conditional recommendation, very low certainty of evidence stemming from narrative review).
25) In patients with RA-ILD being followed-up, when should HRCT be repeated?	We suggest regularly repeating HRCT after 1–2 years in patients with RA-ILD, particularly in those at higher risk of progression and in case of suspected progression (conditional recommendation, very low certainty of evidence stemming from narrative review).
26) In patients with IIM-ILD being followed-up, when should HRCT be repeated?	We suggest repeating HRCT after 3–6 months in patients at risk of developing severe or rapidly progressive IIM-ILD, annually over the first 2 years, and in case of suspected progression (conditional recommendation, very low certainty of evidence stemming from narrative review).
27) In patients with other CTD-ILD being followed-up, when should HRCT be repeated?	We suggest repeating HRCT after 1–2 years in patients with other CTD-ILD, particularly in those at higher risk of progression and in case of suspected progression (conditional recommendation, very low certainty of evidence stemming from narrative review).
Treatment
28) Can key factors be used for the choice of treatment for CTD-ILD?	We suggest using the inclusion criteria of RCTs to guide treatment decisions for CTD-ILD (conditional recommendation, very low certainty of evidence).

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SSc: systemic sclerosis; HRCT: high-resolution computed tomography; IIM: idiopathic inflammatory myopathies; anti-CCP: anti-cyclic citrullinated peptide; anti-MDA-5: anti-melanoma differentiation-associated gene 5; MCTD: mixed connective tissue disease; SjD: Sjögren disease; SLE: systemic lupus erythematosus; PFT: pulmonary function test; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; ATA-I: anti-topoisomerase I antibody; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; UIP: usual interstitial pneumonia; BAL: bronchoalveolar lavage; 6MWT: 6-min walk test; PROM: patient-reported outcome measure; FEV₁: forced expiratory volume in the 1 s; RCT: randomised controlled trial.

TABLE 1d.

Future research priorities and research agenda for connective tissue disease (CTD)-associated interstitial lung disease (ILD) and rheumatoid arthritis (RA)-associated ILD

PICO/narrative question	Future research priorities
Should PFTs (FVC, D_LCO) be used as a replacement for HRCT to screen for ILD in patients diagnosed with CTD?	Assess impact of screening with HRCT and PFTs over HRCT alone Assess impact of screening of asymptomatic patients using clinical characteristics and/or biomarkers Assess impact of early screening and diagnosis of ILD on outcomes
Should LUS be used as a replacement for HRCT to screen for ILD in patients diagnosed with CTD?	Develop standardised screening protocols and validate them in prospective studies of asymptomatic patients, and organise educational and training programmes for LUS
Which patients with CTD should be screened for ILD?	Identify novel risk factors, biomarkers or composite measures associated with ILD in CTDs Explore genetic testing (i.e. MUC5B promoter risk variant) as a screening tool in RA patients Assess impact of screening of RA, IIM and SjD patients with risk factors on outcome Conduct multicentre, prospective investigations to determine the prevalence of ILD in all subtypes of other CTDs and risk factors predictive of the development of ILD
How often should patients with CTD be screened for ILD?	Conduct multicentre, prospective investigations to determine the incidence of ILD in each CTD after initial negative screening and the risk factors predictive of development of ILD over time
How should patients be evaluated at baseline to assess risk of ILD progression or death?	Conduct research studies, including large prospective multicentre design studies, to further assess how to define CTD-ILD progression Develop a risk stratification tool to assess risk of CTD-ILD progression and to tailor differential upfront treatment strategies depending on risk of poor outcomes Clarify the impact of RA activity on the prognosis of RA-ILD In IIM: `-` Investigate and validate prognostic biomarkers in IIM-ILD across different ethnicities, as most published studies have been carried out in Asian cohorts `-` Further evaluate HRCT scores (including quantitative scores) to assess severity and predict risk of ILD progression in patients with anti-MDA-5 or anti-synthetase autoantibodies `-` Standardise myositis autoantibody assays
What is the role of BAL and/or lung biopsy in patients with CTD-ILD at the time of diagnosis?	Assess novel biomarkers in BAL Evaluate the use of cryobiopsy to better categorise fibrotic patterns Evaluate the use of genomic subclassification via transbronchial lung biopsy Assess the application of liquid biopsy in CTD-ILD in clinical practice
What is the role of the 6MWT in assessing severity and/or prognosis of ILD in patients with CTD?	Conduct prospective studies to assess the role of the 6MWT in each CTD, including the prognostic role of: 1) desaturation thresholds and distance walked; 2) heart rate and dyspnoea recovery time, and blood pressure response; and 3) response to oxygen during the 6MWT in patients who experience oxygen desaturation Explore the role of the “1-min sit to stand” test and whether it is a suitable alternative to the more cumbersome requirements of the 6MWT Assess the role of remote 6MWT evaluation (i.e. at home, using mobile devices) Especially for IIM, assess the prognostic impact of the 6MWT in the different stages of IIM-ILD, including in patients with rapidly evolving lung involvement.
What is the role of PROMs in assessing severity and/or prognosis of ILD in patients with CTD?	Assess the association between PROMs and traditional severity measurements (e.g. HRCT, PFTs) Evaluate the optimal frequency of PROM completion
In patients with CTD-ILD being followed-up, when should PFTs and HRCT be repeated?	Conduct large prospective research studies with protocoled regular PFTs and chest HRCT at predefined intervals to establish an evidence-based algorithm for monitoring and risk stratification, and to decide on HRCT versus PFTs versus combination of both for long-term follow-up Determine the ideal time intervals in each CTD depending on: 1) acute versus subacute versus chronic onset; 2) rapidly progressive versus chronic course of disease; and 3) different ILD subtypes; particularly in IIM this should be assessed depending also on different IIM subtypes and/or autoantibody status Assess the role automated quantification of changes on HRCT Assess the role of artificial intelligence for disease trajectory prediction Assess the role of home-monitoring tools of lung function, oximetry and heart rate Evaluate the ideal lung function parameters as outcome measures (FVC only, D_LCO only, combination/others) In all, but particularly in SSc, assess the utility of repeating TLC measurements on follow-up
*Should immunosuppressive treatment versus* control be used for CTD-ILD?**	Identify and develop markers/tools for early detection and prediction of progression to enable timely treatment in patients at risk Assess the molecular landscape to identify dysregulated pathways in SSc-ILD, including identification of disease-specific endotype targets Assess long-term patient experiences and side-effects with acceptability (registry) studies in real-life cohorts Assess 1) safety and efficacy of combination treatment (anti-fibrotic and immunosuppressive treatments); 2) novel treatment modalities, including CAR-T cells and bi-/tri-specific antibodies; and 3) MMF versus placebo in patients with very limited ILD in RCTs Assess the efficacy of tocilizumab across the SSc-ILD population outside of early inflammatory SSc Perform a large RCT of rituximab versus standard of care or other immunosuppressant Conduct RCTs comparing the different treatment options in patients with RA-ILD Evaluate in prospective collaborative studies the safety and efficacy of immunosuppressants in patients with SjD-, MCTD- and SLE-ILD; outcomes should include PFTs, HRCT, survival, symptoms and quality of life, with a long-term perspective Specific topics for IIM-ILD include: `-` Conduct RCTs allowing background therapy `-` Identify the best treatment options for the different subgroups of IIM `-` Evaluate disease specific PROMs of immunosuppressants in IIM-ILD and novel treatment modalities, including CAR-T cells, bi-/tri-specific antibodies, and plasmapheresis `-` Conduct randomised clinical trials of IVIG versus placebo for the treatment of ILD
*Should anti-fibrotic medication versus* control be used for patients with CTD-ILD?**	On top of general priorities, assess effects of pirfenidone and nintedanib on mortality and long-term outcomes Assess efficacy of pirfenidone in patients with different HRCT patterns (UIP versus NSIP)
*Should combination therapy versus* control be used for patients with CTD-ILD?**	Assess efficacy and safety studies of novel agents in combination, both upfront combination and sequential to prevent ILD progression and lung damage Evaluate safety, efficacy, side-effects and acceptability of combination treatment with anti-fibrotics and immunosuppressants or combined immunosuppressants or immunosuppressants with glucocorticoids, including evaluation on the lung and other organ manifestations Evaluate long-term patient experience data, effect on quality of life and symptom/side-effects burden
Non-pharmacological management	The role of non-pharmacological management has not been addressed in this guideline but is of great importance Evaluate lung transplant across CTD-ILD populations Determine the role of oxygen use across CTD and RA-ILD populations Assess the role of palliative measures
Can key factors be used for the choice of treatment for CTD-ILD?	Identify and develop key factors for choice of treatment for ILD in CTD-ILD to enable timely treatment with the right intervention on an individual level, including analysis of registry data Determine the safety and efficacy of specific interventions using key factors for choice of treatment for ILD in CTD-ILD in clinical trials

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SSc: systemic sclerosis; PICO: Patients, Intervention, Comparison, Outcomes; PFT: pulmonary function test; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; LUS: lung ultrasound; IIM: idiopathic inflammatory myopathies; SjD: Sjögren disease; anti-MDA-5: anti-melanoma differentiation-associated gene 5; BAL: bronchoalveolar lavage; 6MWT: 6-min walk test; PROM: patient-reported outcome measure; TLC: total lung capacity; CAR-T cell: chimeric antigen receptor T-cell; MMF: mycophenolate mofetil; RCT: randomised controlled trial; MCTD: mixed connective tissue disease; SLE: systemic lupus erythematosus; IVIG: intravenous immunoglobulin; UIP: usual interstitial pneumonia; NSIP: non-specific interstitial pneumonia.

Literature review

Systematic literature searches were conducted for all questions. One information specialist designed and conducted the literature searches in multiple databases (PubMed, Cochrane Library and ClinicalTrials.gov), covering the years until 2022 (supplementary file 1). The reference lists of relevant systematic reviews and meta-analyses were also screened for relevant studies by the task force members, and some additional studies were included. Due to the excessive workload no update of the searches was conducted, but papers until December 2024 which the task force members deemed to be important were included. Screening of the identified studies was conducted by two task force members for each question; disagreements were resolved by a third member. Screening was conducted in two phases (title and abstract screening, and full-text screening). Inclusion and exclusion criteria, as well as the PRISMA flow diagrams [6], for each question are presented in supplementary file 1. For PICO questions, whenever available, randomised controlled trials (RCTs) were the main body of evidence; if these were not available, observational studies were chosen. In absence of direct evidence (RCTs and observational studies), indirect evidence was used to formulate recommendations, if possible, as explained in supplementary file 1. If deemed appropriate by the task force members, evidence was extrapolated from one CTD to another to support formulating recommendations. For treatment PICO questions the task force agreed to not extrapolate from one disease to another.

Evidence syntheses and assessment of the certainty of evidence

PICO questions were assessed via full systematic review, including risk of bias assessment and grading of the evidence [7]. Narrative questions were assessed via systematic literature searches and narrative review of the evidence [4]. If appropriate, meta-analyses were conducted using RevMan 5.4 software [8]. The certainty of evidence was rated using the GRADE approach. For each PICO, GRADE evidence profiles were formulated using the GRADEpro tool (www.gradepro.org/) (supplementary file 2) [7]. The final certainty of evidence for each question was rated as high, moderate, low or very low.

Formulating recommendations

All PICO and narrative questions were phrased by all task force members. For both PICO and narrative questions, the Evidence to Decision (EtD) framework was used to transparently document the process of making recommendations (supplementary file 2). An overview of PICO and narrative questions, explanation, consideration and remarks is given in table 2 and for treatment in the treatment section. Recommendations were graded as strong or conditional [9–11]. The EtD tables and recommendations, strength and direction of the recommendation were discussed in panel meetings, and consensus was reached. The recommendations were phrased using “recommend” and “should” for strong recommendations and “suggest” and “could” for conditional recommendations. We prepared algorithms covering screening, diagnosis, monitoring and treatment based on the PICO and narrative questions, and added usual clinical practice. The chairs drafted the manuscript, which was then reviewed and approved by all co-authors.

TABLE 2.

Overview of PICO (Patients, Intervention, Comparison, Outcomes) and narrative questions (NQs), explanation, considerations and remarks for screening, diagnosis and monitoring for connective tissue disease (CTD)-associated interstitial lung disease (ILD) and rheumatoid arthritis (RA)-associated ILD

	Explanation	Considerations and remarks
PICO 1–4: Should PFTs (FVC and D_LCO) be used as a replacement for HRCT to screen for ILD in patients diagnosed with CTD?	We assessed whether PFTs could replace HRCT as a screening tool for ILD in patients with CTDs. The populations were patients diagnosed with the respective CTD (SSc, RA, IIM or other CTDs), the intervention PFTs (FVC or D_LCO), the comparator HRCT and the outcome was the identification of ILD.	Although PFTs should not replace HRCT for screening, PFTs and assessment of respiratory symptoms are deemed important for functional and symptomatic assessment.
PICO 5–11: Should LUS be used as a replacement for HRCT to screen for ILD in patients diagnosed with CTD?	We assessed whether LUS could replace HRCT as a screening tool for ILD. The protocols of LUS found in the literature were divided into extended protocol, where the sonographers quantified B-lines (lung comet tails) in more than 18 intercostal spaces bilaterally, and the short protocol, where the quantification was conducted in 18 or fewer intercostal spaces bilaterally. Seven PICOs were formulated, where the population were patients diagnosed with CTDs (generally CTDs, SSc, RA or other CTDs including SjD, IIM and SLE), the intervention LUS (extended and/or short protocol), the comparator HRCT and the outcome the identification of ILD.	LUS can be performed at the bedside, is low-cost, non-invasive, and without radiation for the patient. However, it requires well-trained operators, the examination and its results are highly user-dependent, and B-lines are not specific for ILD.
NQ 1–6: Which patients with CTD should be screened for ILD?	The NQ assessed whether risk factors exist that could enable selection of patient populations for screening of ILD. Predefined risk factors included extrapulmonary CTD features, demographic factors, genetics, constitutional symptoms, clinical examination, the presence of some clinical biomarkers (e.g. autoantibodies, inflammatory markers) and environmental factors (e.g. smoking). The outcome was presence of ILD on HRCT.
NQ 7: How often should patients with CTD be screened for ILD?	The NQ addressed how often CTD patients should be screened after initial negative screening to detect incident ILD. Studies assessing the frequency of development of incident ILD on HRCT were included.
NQ 8–11: How should patients with CTD-ILD be evaluated at baseline to assess risk of ILD progression or death?	These NQs addressed whether risk factors exist to assess risk of worse outcomes, including risk of progression and death. Only baseline factors currently in widespread clinical use were included. Novel and/or not widely available biomarkers were excluded.	Remarks and considerations for all sub-questions: The risk factors addressed below are associated with worse outcome at a population level, recognising that it is not possible in any individual patient to confidently exclude the risk of progression or death.
NQ 12–13: What is the role of BAL and/or lung biopsy in patients with CTD-ILD at time of diagnosis?	The following NQs address the utility of BAL and/or lung biopsy at the time of diagnosis for patients diagnosed with ILD on HRCT.	BAL and lung biopsy are invasive and thus may be uncomfortable for the patient, and may associate with complications, including acute ILD exacerbation, bleeding and pneumothorax, which in specific cases can lead to worsening of morbidity and mortality. Thus, a multidisciplinary team discussion for individual cases is suggested to decide if BAL or lung biopsy is required, to balance risks and benefits and to decide how the result of these examinations might alter management. In cases of differential diagnosis including suspected malignancy, lung biopsy might be needed, while especially in low-grade lymphomas BAL clonality and diagnostic flow cytometry might be adequate for the diagnosis without tissue.
NQ 14–17: What is the role of the 6MWT in assessing severity and/or prognosis of ILD in patients with CTDs?	This NQ addressed whether 6MWT, including oxygen desaturation on exertion, has a role in the assessment of severity and/or as a prognostic marker in patients diagnosed with CTD-ILD.	CTD-ILD patients often have extrapulmonary organ manifestations or treatment-related factors (e.g. glucocorticoids), which can influence the 6MWT, such as significant skin fibrosis, vascular and musculoskeletal impairment in SSc; lower limb joint deformity, active synovitis and/or structural damages resulting from synovitis in RA; significant musculoskeletal involvement (severe myalgia, muscle weakness, arthritis) in IIM; and specific CTD manifestations, such as severe myalgia, muscle weakness and arthritis in other CTDs. The coexistence of pulmonary hypertension may also influence the performance of the 6MWT and oxygen desaturation.
NQ 18–19: What is the role of PROMs in assessing severity and/or prognosis of ILD in patients with CTDs?	The NQ addressed whether PROMs to assess symptom and disease burden and patient needs have a role in the assessment of severity and/or as a prognostic marker in patients diagnosed with CTD-ILD. We included any identified PROM.	Clinicians should not rely only on results of PROMs to assess severity and/or prognosis of ILD in patients with CTD, as associations between PROMs and traditional severity and/or prognostic measurements (such as HRCT and PFTs) are weak. However, PROMs may complement other severity and/or prognosis assessments as they reflect a different and patient-centred domain. Due to the various PROMs assessed, no specific PROMs can be recommended or suggested.
NQ 20–23: In patients with CTD-ILD being followed-up, when should PFTs be repeated?	These NQs address the timeframe in which PFTs should be repeated for the monitoring of patients diagnosed with CTD-ILD.	FVC is considered the most accurate measurement for follow-up of ILD, although fall in D_LCO may also reflect progression of ILD. An isolated worsening in D_LCO may signify concomitant development of pulmonary hypertension. Therefore, both measurements should be monitored in follow-up.
NQ 24–27: In patients with CTD-ILD being followed-up, when should HRCT be repeated?	These NQs addressed the timing in which HRCT should be repeated for monitoring of patients diagnosed with CTD-ILD, as well as the time intervals between repeat follow-up HRCT.	HRCT may also be useful to rule out other causes of worsening of patients, e.g. infections or cancer. Repeating HRCT instead of PFTs may be considered in critically ill patients not able to perform PFTs. For repeat HRCT as regular follow-up, low dose radiation protocols may be considered.

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PFT: pulmonary function test; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; SSc: systemic sclerosis; IIM: idiopathic inflammatory myopathies; LUS: lung ultrasound; SjD: Sjögren disease; SLE: systemic lupus erythematosus; BAL: bronchoalveolar lavage; 6MWT: 6-min walk test; PROM: patient-reported outcome measure.

Screening for ILD in CTDs

General considerations

ILD is a frequent manifestation and associates with high morbidity and mortality across CTDs. In this guideline, we included RA under the umbrella term CTD. Screening is important for early diagnosis of ILD, enabling early interventions. Optimal screening tools and guidance on how often patients should be screened are important.

PICO questions 1–4

Should pulmonary function tests (PFTs) (forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (D_LCO)) be used as a replacement for high-resolution computed tomography (HRCT) to screen for ILD in patients diagnosed with SSc, RA, IIM or other CTD-ILD?

Recommendation

We recommend against replacing HRCT with pulmonary function tests for screening of ILD in patients with SSc, RA, IIM and other CTDs (strong recommendation against, low certainty of evidence for SSc, RA and IIM, and very low certainty of evidence (no studies included) for other CTDs).

Summary of evidence

For SSc: In nine observational studies [12–20], including 1696 SSc patients with ILD and 1495 without ILD, the sensitivity varied between 34% and 92%, and the specificity between 38% and 96%. In several studies, PFTs were inaccurate, missing between 6% and 88% of patients with ILD. The number of correctly identified cases (true-positives) ranged from 190 to 480 per 1000 patients tested, assuming a pre-test probability of 50%. Missed cases of ILD (false-negatives) varied between 20 and 310 per 1000 patients. The number of correctly identified cases (true-negatives) ranged from 170 to 460 per 1000, while false-positive cases ranged from 40 to 330 per 1000 patients. There was risk of bias and inconsistency, leading to an overall low certainty of evidence.

Three observational studies [13, 58, 61] were identified that included 1454 SSc patients. ILD at baseline was observed in 674 (46%) and 39 developed ILD over the 12-month follow-up period. One study found that ILD was detected in all patients at SSc diagnosis [13], and another study showed that only a small proportion of SSc patients developed incident ILD after negative initial screening [58]. The third study noted varying HRCT screening practices across centres [61]. No relevant evidence on screening frequency for other CTDs was demonstrated in any of the studies.

Screening algorithm

Based on the PICO questions 1–11 and narrative questions 1–7, a screening algorithm for ILD detection in CTDs based on the identification of risk factors (table 3) was developed (figure 1).

TABLE 3.

Risk factors in patients with connective tissue disease and rheumatoid arthritis (RA) defining an at-risk patient population that should be screened for interstitial lung disease

	SSc	RA	IIM	SjD
Demographics	Longer disease duration	Older age Male sex Smoking	Older age	Older age Male sex
Circulating markers	Increased KL-6 Presence of ATA-I	Increased ESR Presence of anti-CCP, RF	Increased CRP, ESR Presence of anti-Jo1, anti-MDA-5, anti-Ro52	Increased CRP Presence of anti-Ro52
Extrapulmonary involvement	Diffuse cutaneous SSc Higher mRSS	Higher articular disease activity	Anti-synthetase syndrome Clinical amyopathic dermatomyositis Skin involvement^# Arthritis/arthralgia	Presence of extrapulmonary involvement

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All risk factors were reported in at least two studies; risk factors reported in fewer than two studies are not included. ^#: mechanic's hands, skin ulceration, heliotrope rash. SSc: systemic sclerosis; RA: rheumatoid arthritis; IIM: idiopathic inflammatory myopathies; SjD: Sjögren disease; KL-6: Krebs von den Lungen 6; ATA-I: anti-topoisomerase I antibody; ESR: erythrocyte sedimentation rate; anti-CCP: anti-cyclic citrullinated peptide antibody; RF: rheumatoid factor; CRP: C-reactive protein; anti-Jo1: anti-antihistidyl transfer RNA synthetase antibody; anti-MDA-5: anti-melanoma differentiation-associated protein 5 antibody; mRSS: modified Rodnan skin score.

A screening algorithm for interstitial lung disease (ILD) detection in connective tissue disease (CTD) and rheumatoid arthritis (RA) based on the identification of risk factors. SSc: systemic sclerosis; MCTD: mixed connective tissue disease; IIM: idiopathic inflammatory myopathies; SjD: Sjögren disease; HRCT: high-resolution computed tomography; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide. ^#: IIM: anti-synthetase syndrome, clinical amyopathic dermatomyositis, presence of mechanic's hands, arthritis, anti-synthetase, anti-melanoma differentiation-associated gene 5 and anti-Ro52 antibodies. ^¶: IIM: except patients with inclusion body myositis. ⁺: RA: older age, smoking history, elevated rheumatoid factor, anti-cyclic citrullinated peptide antibodies, increased inflammatory markers, male sex and high RA disease activity. ^§: SjD: older age, active extrapulmonary organ involvement and increased inflammatory markers.

The algorithm for the assessment of patients at the time of diagnosis of ILD on HRCT is based on narrative questions 8–19 (figure 2 and table 4).

TABLE 4.

Risk factors for poor outcome, defined as disease progression and death, in patients with connective tissue disease (CTD)-associated interstitial lung disease (ILD) and rheumatoid arthritis (RA)-associated ILD

	SSc^#	RA^#	IIM^#^,^¶
Demographics	Older age Male sex African American ethnicity	Older age at RA onset Male sex
Circulating markers	Elevated ESR, CRP ATA-I	Anti-CCP, RF	Elevated ferritin Anti-MDA-5, anti-synthetase
Pulmonary function/markers	Baseline PFTs (FVC, D_LCO)	Baseline PFTs (low FVC and/or D_LCO)
Imaging/histology	Higher extent of ILD on HRCT	UIP and probable UIP HRCT/histological patterns Higher extent of ILD on HRCT	Higher extent of ILD on HRCT and ILD pattern on HRCT
Extrapulmonary involvement	Recent onset of SSc with rapid skin progression, more extensive skin fibrosis (mRSS)	Higher articular disease activity

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All risk factors were reported in at least two studies; risk factors reported in fewer than two studies are not included. ^#: combination of several risk factors, such as older age and more extensive ILD on chest HRCT, have been shown to be more associated with ILD progression. As far as other CTDs are concerned, only one study regarding Sjögren disease was found, and thus not included in the table. ^¶: risk factors for poor outcome in anti-MDA-5-positive IIM-ILD patients: extent of ILD on HRCT, higher ferritin levels, KL-6, older age, high P_A−aO₂. SSc: systemic sclerosis; IMM: idiopathic inflammatory myopathies; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; ATA-I: anti-topoisomerase I antibody; anti-CCP: anti-cyclic citrullinated peptide antibody; RF: rheumatoid factor; anti-MDA-5: anti-melanoma differentiation-associated protein 5 antibody; PFT: pulmonary function test; FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; UIP: usual interstitial pneumonia; mRSS: modified Rodnan skin score; KL-6: Krebs von den Lungen 6; P_A−aO₂: alveolar to arterial oxygen tension difference.

The monitoring algorithms are based on narrative questions 14–27 (figure 3a–d).

FIGURE 3b — Monitoring approach for patients with rheumatoid arthritis (RA)-associated interstitial lung disease (ILD). FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWD: 6-min walk distance.

FIGURE 3c — Monitoring approach for patients with interstitial lung disease (ILD) associated with idiopathic inflammatory myopathies (IIM). FVC: forced vital capacity; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWD: 6-min walk distance.

FIGURE 3d — Monitoring approach for patients with interstitial lung disease (ILD) associated with Sjögren disease (SjD) and mixed connective tissue disease (MCTD). CTD: connective tissue disease; FVC: forced vital capacity; FEV₁: forced expiratory volume in 1 s; D_LCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; 6MWD: 6-min walk distance.

Our guideline provides comprehensive support for clinicians and patients, encompassing screening, diagnosis, monitoring and treatment strategies for people with CTD-ILD. Many of the pre-defined management areas are addressed specifically for the underlying rheumatic disease, which is of importance due to the inherent differences across the diseases.

We recommend screening all patients with SSc, MCTD and IIM patients with risk factors using HRCT at time of diagnosis. We suggest that most patients with IIM without risk factors except for inclusion body myositis as well as patients with RA and SjD who have risk factors for ILD should be screened for ILD using HRCT. We suggest that lung biopsy does not play a role in the diagnosis of CTD-ILD but might be considered in an multidisciplinary team discussion if alternative diagnoses are considered [339]. In the case of possible alternative diagnoses or co-existing conditions, BAL could be considered together with other assessments to rule out differential diagnoses, but does not routinely play a role in the diagnosis of ILD in CTDs. We suggest that, at the time of ILD diagnosis, all patients should be assessed for disease severity and risk of progression by performing a risk factor assessment, as well as PFTs, HRCT, 6MWT and PROMs.

For monitoring all CTD-ILD patients, we suggest conducting a comprehensive evaluation during each visit to identify individuals at high or low risk for poor prognosis, ILD progression, and disease severity. Clinical risk factors can be used to identify patients at high or low risk of progression. High risk patients are suggested to be followed with lung function tests every 3–6 months early in the disease course and every 6–12 months thereafter. A control HRCT is suggested to be conducted routinely after 12 months, followed by annual HRCT if clinically indicated. The 6MWT in patients without physical limitations and PROMs are suggested to be conducted every 6–12 months in usual clinical practice. PROMs can provide valuable insights into the patient's perspective, capturing respiratory symptoms in a validated and standardised way, and assessing the impact of the disease on daily life and patient well-being, which can enhance the overall assessment of disease severity and treatment effectiveness. Patients at lower risk are suggested to be followed with the same assessments but over longer time intervals.

The treatment recommendations are comprehensive and summarised per disease, including key factors for choice of treatment in the provided treatment algorithms. In summary, therapy choice should consider patient-specific risk factors, extrapulmonary organ manifestations, potential side-effects, and the risk of progressive or severe ILD. Early and/or aggressive treatment is recommended for patients at risk of progressive or severe ILD, especially those with multiple risk factors or specific indicators like anti-MDA-5 autoantibodies. Specifically, for SSc-ILD, it is suggested to use MMF, rituximab and cyclophosphamide. For early diffuse cutaneous SSc characterised by increased inflammatory markers or recent skin fibrosis progression, tocilizumab is recommended. Nintedanib is suggested for SSc-ILD patients, as well as the combination of nintedanib and MMF (figure 4a). Regarding RA-ILD, immunosuppressive treatment, and the use of pirfenidone for patients displaying a UIP pattern, are suggested (figure 4b). In IIM-ILD, immunosuppressive treatment is recommended. Moreover, the use of combination therapy involving immunosuppressants and glucocorticoids is suggested (figure 4c). In other CTD-ILDs, including SjD-, MCTD- and SLE-ILD, immunosuppressive treatment is suggested. Nintedanib and combination therapy is suggested for any CTD-ILD patients who exhibit progressive pulmonary fibrosis (figure 4d). In addition to pharmacological therapy, we encourage adhering to non-pharmacological treatment and existing specific rheumatic disease recommendations in addition to our ILD-specific guideline to guarantee an optimised and holistic disease management [340, 341].

FIGURE 4a — Treatment algorithms for patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD). HRCT: high resolution computed tomography; MMF: mycophenolate mofetil.

FIGURE 4b — Treatment algorithms for patients with rheumatoid arthritis (RA)-associated interstitial lung disease (ILD). UIP: usual interstitial pneumonia; JAK: Janus kinase.

FIGURE 4c — Treatment algorithms for patients with interstitial lung disease (ILD) associated with idiopathic inflammatory myopathies (IIM). CNI: calcineurin inhibitor; IVIG: intravenous immunoglobulins; JAK: Janus kinase.

FIGURE 4d — Treatment algorithms for other connective tissue disease (CTD)-associated interstitial lung disease (ILD) patients, such as Sjögren disease-, mixed connective tissue disease- or systemic lupus erythematosus-associated ILD.

Our guideline has some limitations. Our guideline predominantly builds on evidence of low and very low certainty. This is a common challenge for rare diseases, attributable to limited patient populations and a scarcity of RCTs with adequate numbers of participants needed to achieve a high level of evidence. This led to most recommendations being conditional. Consequently, we deem our research agenda highly significant and advocate undertaking studies to address these gaps. Using the GRADE methodology agreed on by both societies did not allow us to record level of agreement and this is the reason why this is not reported. Lastly, our guideline is only developed for adult patients, and some aspects may be different in childhood populations.

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Footnotes

This paper was jointly developed by Annals of the Rheumatic Diseases and the European Respiratory Journal, and jointly published by Elsevier BV and the European Respiratory Society. The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style. Either citation can be used when citing this article.

This document was developed by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR).

This document was endorsed by the ERS Executive Committee on 9 June 2025, by the EULAR Council on 16 December 2024, and by ERN-LUNG on 5 June 2025.

The guidelines published by the European Respiratory Society (ERS) incorporate data obtained from a comprehensive and systematic literature review of the most recent studies available at the time. Health professionals are encouraged to take the guidelines into account in their clinical practice. However, the recommendations issued by this guideline may not be appropriate for use in all situations. It is the individual responsibility of health professionals to consult other sources of relevant information, to make appropriate and accurate decisions in consideration of each patient's health condition and in consultation with that patient and the patient's caregiver where appropriate and/or necessary, and to verify rules and regulations applicable to drugs and devices at the time of prescription.

Conflict of interest: K.M. Antoniou reports grants from Boehringer Ingelheim, F. Hoffmann-La Roche Ltd and Menarini, consulting fees from Boehringer Ingelheim, F. Hoffmann-La Roche Ltd and GlaxoSmithKline, support for attending meetings from Chiesi, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, F. Hoffmann-La Roche Ltd and GlaxoSmithKline. O. Distler reports grants from Kymera, Mitsubishi Tanabe and Boehringer Ingelheim, consulting fees from 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant Sciences, Amgen, AnaMar, Area, Arxx, AstraZeneca, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catalyze Capital, Corbus Pharmaceuticals, CSL Behring, EMD Serono, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Nkarta Inc., Novartis, Orion, Prometheus Biosciences, Quell Therapeutics, Redxpharma, Roivant, Topadur and UCB, payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Boehringer Ingelheim, Janssen and Medscape, patents planned, issued or pending (mir-29 for the treatment of systemic sclerosis), leadership roles with FOREUM Foundation, ERS/EULAR Guidelines, EUSTAR, SCQM, Swiss Academy of Medical Sciences (SAMW) and the Hartmann Müller Foundation, and is co-founder of Citus AG. A-M. Gheorghiu reports grants from Foundation for Research in Rheumatology (FOREUM), consulting fees from Boehringer Ingelheim, payment or honoraria for lectures, presentations, manuscript writing or educational events from Abbvie, Boehringer Ingelheim, Novartis, Sandoz and Sobi, support for attending meetings from Ewopharma, Boehringer Ingelheim and Janssen, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. C.C. Moor reports grants from Boehringer Ingelheim, AstraZeneca and Daiichi Sankyo, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, AstraZeneca and GSK, and leadership roles with European Respiratory Society, European Lung Foundation and Advocacy Council of the ERS. J. Vikse reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Abbvie, Boehringer Ingelheim, GSK, Janssen, Merck, Novartis, ThermoFisher and UCB, and participation on a data safety monitoring board or advisory board with Novartis. E. Bargagli reports grants from Chiesi and AstraZeneca, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim. Y. Allanore reports grants from Alpine Immunosciences, Merck Serono, Corvus and Medsenic, consulting fees from Boehringer Ingelheim, Abbvie, AstraZeneca, Novartis, Argenx, Horizon and Topadur, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, and support for attending meetings from Celltrion and AstraZeneca. T.J. Corte reports grants from Avalyn Pharma, Boehringer Ingelheim, Pharmaxis, Bristol Myers Squibb, 4D, Roche, Pliant, Bridge Biotherapeutics and Avalyn Therapeutics, consulting fees from Boehringer Ingelheim, Pharmaxis, Bristol Myers Squibb, Ad Alta, Roche, Pliant, Bridge Biotherapeutics, Avalyn Therapeutics, DevPro and Endeavour BioMedicine, payment or honoraria for lectures, presentations, manuscript writing or educational events from Bristol Myers Squibb, Roche and Boehringer Ingelheim, support for attending meetings from Bristol Myers Squibb and Boehringer Ingelheim, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Ad Alta, Bristol Myers Squibb, Roche, Pliant, Bridge Biotherapeutics, Avalyn Therapeutics, DevPro and Endeavour BioMedicine. P. Dieudé reports grants from Bristol Myers Squibb and Pfizer, consulting fees from Boehringer Ingelheim, Bristol Myers Squibb, Janssen, Abbvie and Pfizer, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Bristol Myers Squibb, Janssen, Abbvie and Pfizer, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Bristol Myers Squibb and Pfizer. V. Cottin reports consulting fees from Abbvie, AstraZeneca, Avalyn, Boehringer Ingelheim, BMS/Celgene, CSL (Behring, Vifor), Ferrer/United Therapeutics, Gossamer, GSK, Liquidia, Pliant, PureTech, Roche, Roivant, Sanofi and Shionogi, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Ferrer/United Therapeutics, Roche and Sanofi, support for attending meetings from Boehringer Ingelheim and Sanofi, participation on a data safety monitoring board or advisory board with GSK and Molecure, and a leadership role (on an adjudication committee) with Fibrogen. B.A. Fisher reports grants from Janssen, Servier, Galapagos and Celgene, consulting fees from Novartis, Roche, BMS, Janssen, UCB, Sanofi, Servier, Galapagos, AstraZeneca, Otsuka, Amgen and Kiniksa, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Novartis and Servier. J.T. Giles reports consulting fees from AbbVie, Pfizer, Eli Lilly, Novartis, Merck and Sana, and participation on a data safety monitoring board or advisory board with Janssen. M. Kreuter reports consulting fees from GSK, Boehringer Ingelheim, AstraZeneca, Pliant, Roche, BMS, Trevi and Galapagos, and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim. I.E. Lundberg reports grants from Swedish Research Council (2020-01378), Region Stockholm (ALF project), Swedish Rheumatism Association, King Gustaf V 80 Year Foundation, Heart and Lung Foundation, Anna Hedin Foundation and AstraZeneca, consulting fees from Chugai Pharmaceutical Co. Ltd, Bristol Myers Squibb, EMD Serono, Research and Development Institute, Galapagos, Argenx, Pfizer and Janssen, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, and stock or stock options with Novartis and Roche. V. Poletti reports consulting fees from Boehringer Ingelheim and ERBE, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. B. Maurer reports grants from AbbVie, Protagen and Novartis Biomedical Research, consulting fees from Novartis, Boehringer Ingelheim, Janssen-Cilag and GSK, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, GSK, Novartis, Otsuka and MSD, support for attending meetings from Medtalk, Pfizer, Roche, Actelion, Mepha, MSD and Boehringer Ingelheim, patents planned, issued or pending (mir-29 for the treatment of systemic sclerosis), and participation on a data safety monitoring board or advisory board with Janssen and Boehringer Ingelheim. E.A. Renzoni reports grants from Boehringer Ingelheim, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Mundipharma, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. U. Müller-Ladner reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Medac. M.E. Strek reports grants from Boehringer Ingelheim Pharmaceuticals, Inc., NIH and the Pulmonary Fibrosis Foundation, consulting fees from Boehringer Ingelheim Pharmaceuticals, Inc., payment or honoraria for lectures, presentations, manuscript writing or educational events from CHEST and Boehringer Ingelheim Pharmaceuticals, Inc., support for attending meetings from Boehringer Ingelheim Pharmaceuticals, Inc., and participation on a data safety monitoring board or advisory board with Fibrogen, Bristol Myers Squibb and Pliant Therapeutics. P. Studenic reports grants from FOREUM, Horizon/HADEA, AAL, EUREKA and Jubiläumsfond der Stadt Wien, consulting fees from AbbVie, payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca and AbbVie, and support for attending meetings from Janssen and Galapagos. T. Stamm reports grants and personal fees from Roche, consulting fees and payment or honoraria for lectures, presentations, manuscript writing or educational events from AbbVie, Roche, Sanofi, Takeda and Novartis. B. Crestani reports grants from Boehringer Ingelheim, consulting fees from BMS, Boehringer Ingelheim, Chiesi, CSL Behring, GSK and Sanofi, payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, BMS, Boehringer Ingelheim, GSK, Novartis, Roche and Sanofi, support for attending meetings from AstraZeneca, BMS, Boehringer Ingelheim, Roche and Sanofi, participation on a data safety monitoring board or advisory board with BMS, Boehringer Ingelheim, GSK, Horizon and Sanofi, and a leadership role with Fondation du Souffle. A-M. Hoffmann-Vold reports grants from Boehringer Ingelheim and Janssen, consulting fees from Abbvie, Merck Sharp & Dohme, Arxx Therapeutics, Pliant Therapeutics, BMS, Roche, Boehringer Ingelheim, Werfen, Genentech and Janssen, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Janssen, Medscape, Merck Sharp & Dohme, Novartis and Roche, support for attending meetings from Boehringer Ingelheim, and leadership role with the CTD-ILD ERS/EULAR and EULAR study groups on the lung in rheumatic and musculoskeletal diseases. The remaining authors have no potential conflicts of interest to disclose.

Support statement: This work was supported by the European Respiratory Society (grant: ERS Task Force TF-2020-03). Funding information for this article has been deposited with the Open Funder Registry.

Supplementary material

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Supplementary file 1: Methodology and search strategy

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DOI: 10.1183/13993003.02533-2024.Supp1

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Supplementary file 2: GRADE evidence profiles and evidence to decision frameworks

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Pocket guideline

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PERMALINK

ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease

Katerina M Antoniou

Oliver Distler

Ana-Maria Gheorghiu

Catharina C Moor

Jens Vikse

Nikoleta Bizymi

Ilaria Galetti

Graham Brown

Elena Bargagli

Yannick Allanore

Tamera J Corte

Philippe Dieudé

Vincent Cottin

Benjamin A Fisher

Aurelie Fabre

Jon T Giles

Michael Kreuter

Ingrid E Lundberg

Venerino Poletti

Britta Maurer

Elisabetta A Renzoni

Ulf Müller-Ladner

Mary E Strek

Nicola Sverzellati

Paul Studenic

Jibril Mohammed

Blin Nagavci

Tanja Stamm

Thomy Tonia

Bruno Crestani

Anna-Maria Hoffmann-Vold

Abstract

Background

Methods

Results

Conclusions

Shareable abstract

Introduction

Methods

Task force composition

Formulation of questions and selection of outcomes and their importance

TABLE 1a.

TABLE 1b.

TABLE 1c.

TABLE 1d.

Literature review

Evidence syntheses and assessment of the certainty of evidence

Formulating recommendations

TABLE 2.

Screening for ILD in CTDs

General considerations

PICO questions 1–4

Recommendation

Summary of evidence

Justification of recommendation

PICO questions 5–11

Recommendation

Summary of evidence

Justification of recommendation

Narrative question 1

Recommendation

Summary of evidence

Justification of recommendation

Narrative question 2

Recommendation

Remarks and considerations

Summary of evidence

Justification of recommendation

Narrative question 3

Recommendation 1

Recommendation 2

Summary of evidence

Justification of recommendation

Narrative question 4

Recommendation

Summary of evidence

Justification of recommendation

Narrative question 5